Q5BL07 (PEX1_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Peroxisome biogenesis factor 1

Alternative name(s):
Peroxin-1

Gene names

Name:

Pex1

Organism

Mus musculus (Mouse) [Reference proteome]

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus › Mus

Protein attributes

Sequence length	1284 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Required for stability of PEX5 and protein import into the peroxisome matrix. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes By similarity.
Subunit structure	Interacts directly with PEX6. Interacts indirectly with PEX26, via its interaction with PEX6 By similarity.
Subcellular location	Cytoplasm By similarity. Peroxisome membrane By similarity. Note: Associated with peroxisomal membranes By similarity.
Domain	The N-terminal domain shows evolutionary conservation with that of VCP, and is able to bind phospholipids with a preference for phosphatidylinositol monophosphates.
Sequence similarities	Belongs to the AAA ATPase family.

Ontologies

Keywords
Biological process	Peroxisome biogenesis Protein transport Transport
Cellular component	Cytoplasm Membrane Peroxisome
Coding sequence diversity	Alternative splicing
Disease	Peroxisome biogenesis disorder
Domain	Repeat
Ligand	ATP-binding Lipid-binding Nucleotide-binding
PTM	Isopeptide bond Phosphoprotein Ubl conjugation
Technical term	3D-structure Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	microtubule-based peroxisome localization Inferred from electronic annotation. Source: Compara protein import into peroxisome matrix Inferred from electronic annotation. Source: Compara
Cellular_component	cytosol Inferred from electronic annotation. Source: Compara nucleus Inferred from electronic annotation. Source: Compara peroxisomal membrane Inferred from electronic annotation. Source: UniProtKB-SubCell peroxisome Inferred from direct assay PubMed 14561759. Source: HGNC
Molecular_function	ATP binding Inferred from electronic annotation. Source: UniProtKB-KW ATPase activity, coupled Inferred from electronic annotation. Source: Compara lipid binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q5BL07-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 2 (identifier: Q5BL07-2)

The sequence of this isoform differs from the canonical sequence as follows:
     413-452: Missing.

Note: No experimental confirmation available.

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 1284

1284

Peroxisome biogenesis factor 1

PRO_0000304931

Regions

Nucleotide binding

600 – 607

8

ATP Potential

Nucleotide binding

882 – 889

8

ATP Potential

Amino acid modifications

Modified residue

1210

1

Phosphoserine By similarity

Modified residue

1212

1

Phosphoserine Ref.4 Ref.5

Cross-link

834

Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin) By similarity

Natural variations

Alternative sequence

413 – 452

40

Missing in isoform 2.

VSP_028131

Experimental info

Mutagenesis

135

1

R → A: Loss of phospholipid-binding. Ref.3

Mutagenesis

174

1

K → A: No effect on phospholipid-binding. Ref.3

Secondary structure

1

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1284

Helix Strand Turn

Details...

Beta strand

15 – 22

8

Beta strand

25 – 27

3

Beta strand

29 – 31

3

Helix

33 – 38

6

Beta strand

47 – 53

7

Beta strand

56 – 58

3

Beta strand

60 – 62

3

Beta strand

73 – 77

5

Helix

78 – 83

6

Beta strand

91 – 96

6

Beta strand

104 – 112

9

Helix

113 – 121

9

Helix

126 – 133

8

Beta strand

142 – 150

9

Beta strand

152 – 162

11

Beta strand

165 – 168

4

Beta strand

174 – 177

4

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified October 2, 2007. Version 2. Checksum: 29B6C9B6E93FA6EF	FASTA	1,284	141,428
	10 20 30 40 50 60 MWSSDRLAGA GSGGAVVTVA FTNARDCFLH LPRRLVAQLH LLQNQAIEVA SDHQPTYLSW 70 80 90 100 110 120 VEGRHFNDQS ENVAEINRQV GQKLGLSSGD QVFLRPCSHV VSCQQVEVEP LSADDWEILE 130 140 150 160 170 180 LHAISLEQHL LDQIRIVFPK AVVPIWVDQQ TYIFIQIVTL MPAAPYGRLE TNTKLLIQPK 190 200 210 220 230 240 TRQAKESTFP KEGDAHGQVH SYGREQKGLS KELQTRQLHT NSEGITASNG RDPKVPGGPL 250 260 270 280 290 300 KPSWWAVLGS MLSFGPDSKQ ESAWGSLELG AFKNMQSQAA PLEGTFRVCQ VQPPSARTTT 310 320 330 340 350 360 ATSVFHKHCT AHVFPWDQEY FDVEPSFTVT YGKLVKLHSP KQQQDKSKQG VLLPDKEKQL 370 380 390 400 410 420 SKSPDHKQIS SNRSEEAAEA CVLKVVWNGL EELKNATEFT ESLELLHRGK VWIPDDLRKR 430 440 450 460 470 480 LNIEMHAVVR ITPLETTPKI PRSLKLQPRE NLPKDVNEET IKTVFSSWVQ QSATTMLPLV 490 500 510 520 530 540 ISKEERIKLE IKDGLREFSL STVHSQEKEK EEGKTVFVLS SILLQKISVQ VLLEPMIKEE 550 560 570 580 590 600 QSAEIDFLLP SLTLSSLGGV SALGASAMEH ITHSLLGRPL SRQLMALVAG LRNGALLITG 610 620 630 640 650 660 GKGSGKSTFA KAICKEAQDT LDARVETVDC KALRGKRLES IQKALEVAFS EAAWRQPSVI 670 680 690 700 710 720 LLDDLDLIAG LPSVPEQEHS PEAVQSQRLA HALNDMIKEF VSTGSLVALI ATSQLQQSLH 730 740 750 760 770 780 PSLVSAQGIH TFQCVQHLQP PNPEQRCEIL HSVVKNKLGC DISNFPDLDL QCIAKDTEAF 790 800 810 820 830 840 VARDFTVLVD RAIHSSLSRQ HSSSREDLTL TTSDFQKALR GFLPASLRNV NLHKPRDLGW 850 860 870 880 890 900 DKIGGLHEVR QILMDTIQLP AKYPELFANL PIRQRTGILL YGPPGTGKTL LAGVVARESG 910 920 930 940 950 960 MNFISIKGPE LLSKYIGASE QAVRDVFIRA QAAKPCILFF DEFESIAPRR GHDNTGVTDR 970 980 990 1000 1010 1020 VVNQLLTQLD GVEGLQGVYV LAATSRPDLI DPALLRPGRL DKCVYCPPPD QVSRLEILTV 1030 1040 1050 1060 1070 1080 LSKSLALADD VDLQHVASVT DSFTGADLKA LLYNAQLEAL QGRLLPSGLP DGGSSSDSDL 1090 1100 1110 1120 1130 1140 SLSSMVFLNH SSGSDDSAGD GECGLEQSLL SLEMSEILPD ESKFNMYRLY FGSSYESELG 1150 1160 1170 1180 1190 1200 NGTPSDLSSH CLSAPSSVTQ DLPAAPGKDP LFTQHPVFRT PSQEGCQDLT QEQRDQLRAE 1210 1220 1230 1240 1250 1260 ISIIKGRYRS QSGEDESLNQ PGPIKTTFAI SQAHLMTALA HTRPSISEDE GKEFAELYEN 1270 1280 FQNPKKRKNQ SGTVFRTGQK VTLA
	Isoform 2 [UniParc]. Checksum: 9B1073BBA4CC4106	FASTA	1,244	136,743
	10 20 30 40 50 60 MWSSDRLAGA GSGGAVVTVA FTNARDCFLH LPRRLVAQLH LLQNQAIEVA SDHQPTYLSW 70 80 90 100 110 120 VEGRHFNDQS ENVAEINRQV GQKLGLSSGD QVFLRPCSHV VSCQQVEVEP LSADDWEILE 130 140 150 160 170 180 LHAISLEQHL LDQIRIVFPK AVVPIWVDQQ TYIFIQIVTL MPAAPYGRLE TNTKLLIQPK 190 200 210 220 230 240 TRQAKESTFP KEGDAHGQVH SYGREQKGLS KELQTRQLHT NSEGITASNG RDPKVPGGPL 250 260 270 280 290 300 KPSWWAVLGS MLSFGPDSKQ ESAWGSLELG AFKNMQSQAA PLEGTFRVCQ VQPPSARTTT 310 320 330 340 350 360 ATSVFHKHCT AHVFPWDQEY FDVEPSFTVT YGKLVKLHSP KQQQDKSKQG VLLPDKEKQL 370 380 390 400 410 420 SKSPDHKQIS SNRSEEAAEA CVLKVVWNGL EELKNATEFT ESLELLHRGK VWPKDVNEET 430 440 450 460 470 480 IKTVFSSWVQ QSATTMLPLV ISKEERIKLE IKDGLREFSL STVHSQEKEK EEGKTVFVLS 490 500 510 520 530 540 SILLQKISVQ VLLEPMIKEE QSAEIDFLLP SLTLSSLGGV SALGASAMEH ITHSLLGRPL 550 560 570 580 590 600 SRQLMALVAG LRNGALLITG GKGSGKSTFA KAICKEAQDT LDARVETVDC KALRGKRLES 610 620 630 640 650 660 IQKALEVAFS EAAWRQPSVI LLDDLDLIAG LPSVPEQEHS PEAVQSQRLA HALNDMIKEF 670 680 690 700 710 720 VSTGSLVALI ATSQLQQSLH PSLVSAQGIH TFQCVQHLQP PNPEQRCEIL HSVVKNKLGC 730 740 750 760 770 780 DISNFPDLDL QCIAKDTEAF VARDFTVLVD RAIHSSLSRQ HSSSREDLTL TTSDFQKALR 790 800 810 820 830 840 GFLPASLRNV NLHKPRDLGW DKIGGLHEVR QILMDTIQLP AKYPELFANL PIRQRTGILL 850 860 870 880 890 900 YGPPGTGKTL LAGVVARESG MNFISIKGPE LLSKYIGASE QAVRDVFIRA QAAKPCILFF 910 920 930 940 950 960 DEFESIAPRR GHDNTGVTDR VVNQLLTQLD GVEGLQGVYV LAATSRPDLI DPALLRPGRL 970 980 990 1000 1010 1020 DKCVYCPPPD QVSRLEILTV LSKSLALADD VDLQHVASVT DSFTGADLKA LLYNAQLEAL 1030 1040 1050 1060 1070 1080 QGRLLPSGLP DGGSSSDSDL SLSSMVFLNH SSGSDDSAGD GECGLEQSLL SLEMSEILPD 1090 1100 1110 1120 1130 1140 ESKFNMYRLY FGSSYESELG NGTPSDLSSH CLSAPSSVTQ DLPAAPGKDP LFTQHPVFRT 1150 1160 1170 1180 1190 1200 PSQEGCQDLT QEQRDQLRAE ISIIKGRYRS QSGEDESLNQ PGPIKTTFAI SQAHLMTALA 1210 1220 1230 1240 HTRPSISEDE GKEFAELYEN FQNPKKRKNQ SGTVFRTGQK VTLA

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2). Strain: C57BL/6. Tissue: Brain.
[2]	"The transcriptional landscape of the mammalian genome." Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J. , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y. Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 1-505 AND 795-1284 (ISOFORM 1). Strain: C57BL/6J. Tissue: Head and Liver.
[3]	"The common phospholipid-binding activity of the N-terminal domains of PEX1 and VCP/p97." Shiozawa K., Goda N., Shimizu T., Mizuguchi K., Kondo N., Shimozawa N., Shirakawa M., Hiroaki H. FEBS J. 273:4959-4971(2006) [PubMed] [Europe PMC] [Abstract] Cited for: PHOSPHOLIPID BINDING, MUTAGENESIS OF ARG-135 AND LYS-174.
[4]	"Large-scale phosphorylation analysis of mouse liver." Villen J., Beausoleil S.A., Gerber S.A., Gygi S.P. Proc. Natl. Acad. Sci. U.S.A. 104:1488-1493(2007) [PubMed] [Europe PMC] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-1212, MASS SPECTROMETRY. Tissue: Liver.
[5]	"Specific phosphopeptide enrichment with immobilized titanium ion affinity chromatography adsorbent for phosphoproteome analysis." Zhou H., Ye M., Dong J., Han G., Jiang X., Wu R., Zou H. J. Proteome Res. 7:3957-3967(2008) [PubMed] [Europe PMC] [Abstract] Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-1212, MASS SPECTROMETRY. Tissue: Liver.
[6]	"Structure of the N-terminal domain of PEX1 AAA-ATPase. Characterization of a putative adaptor-binding domain." Shiozawa K., Maita N., Tomii K., Seto A., Goda N., Akiyama Y., Shimizu T., Shirakawa M., Hiroaki H. J. Biol. Chem. 279:50060-50068(2004) [PubMed] [Europe PMC] [Abstract] Cited for: X-RAY CRYSTALLOGRAPHY (2.05 ANGSTROMS) OF 3-181.
+	Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ

BC090845 mRNA. Translation: AAH90845.1.
AK017309 mRNA. Translation: BAB30684.1.
AK149599 mRNA. Translation: BAE28984.1.

IPI

IPI00136789.
IPI00653755.

RefSeq

NP_082053.1. NM_027777.1.

UniGene

Mm.379196.

3D structure databases

PDBe
RCSB PDB
PDBj

Entry	Method	Resolution (Å)	Chain	Positions	PDBsum
1WLF	X-ray	2.05	A	3-181	[»]

ProteinModelPortal

Q5BL07.

SMR

Q5BL07. Positions 13-179, 595-1117.

ModBase

Search...

PTM databases

PhosphoSite

Q5BL07.

Proteomic databases

PaxDb

Q5BL07.

PRIDE

Q5BL07.

Protocols and materials databases

StructuralBiologyKnowledgebase

Search...

Genome annotation databases

Ensembl

ENSMUST00000006061; ENSMUSP00000006061; ENSMUSG00000005907.
ENSMUST00000121291; ENSMUSP00000113304; ENSMUSG00000005907.

GeneID

71382.

KEGG

mmu:71382.

UCSC

uc008whf.1. mouse.

Organism-specific databases

CTD

5189.

MGI

MGI:1918632. Pex1.

Phylogenomic databases

eggNOG

COG0464.

GeneTree

ENSGT00550000075032.

HOGENOM

HOG000252959.

HOVERGEN

HBG008169.

InParanoid

Q5BL07.

KO

K13338.

OMA

HSWEKEK.

OrthoDB

EOG4JM7P2.

Gene expression databases

ArrayExpress

Q5BL07.

Bgee

Q5BL07.

CleanEx

MM_PEX1.

Genevestigator

Q5BL07.

Family and domain databases

Gene3D

2.40.40.20. 1 hit.

InterPro

IPR003593. AAA+_ATPase.
IPR009010. Asp_de-COase-like_dom.
IPR003959. ATPase_AAA_core.
IPR003960. ATPase_AAA_CS.
IPR015343. Peroxisome_synth_fac_1_a/b.
IPR015342. PEX-1N.
IPR025653. Pex1.
[Graphical view]

PANTHER

PTHR23077:SF12. PTHR23077:SF12. 1 hit.

Pfam

PF00004. AAA. 2 hits.
PF09262. PEX-1N. 1 hit.
PF09263. PEX-2N. 1 hit.
[Graphical view]

SMART

SM00382. AAA. 2 hits.
[Graphical view]

SUPFAM

SSF50692. Asp_decarb_fold. 1 hit.

PROSITE

PS00674. AAA. 1 hit.
[Graphical view]

ProtoNet

Search...

Other

EvolutionaryTrace

Q5BL07.

NextBio

333669.

SOURCE

Search...

Entry information

Entry name

PEX1_MOUSE

Accession

Primary (citable) accession number: Q5BL07
Secondary accession number(s): Q3UEC7, Q9CU85

Entry history

Integrated into UniProtKB/Swiss-Prot:	October 2, 2007
Last sequence update:	October 2, 2007
Last modified:	May 1, 2013
This is version 78 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program